Follow-up With Cardiology

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The Conclusion from Medical Updates Part 1Part 2Part 2.5,

Part 3: The Cardiac MRI, &  Part 3: The Exercise Stress Test


Although preparing for any type of doctors appointment is stressful enough for almost anyone, it is even harder when you have a chronic illness, or even worse – multiple chronic conditions.

There is always so much preparation and pressure that goes into getting ready for each appointment:
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However, it’s entirely worse when you know you are  awaiting abnormal tests results.

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My list of questions for my cardiologist had gotten so long,

I was afraid to even present it to the cardiologist:

  • Medication questions:
    • Should I take the beta-blockers in the morning or evening?
    • Will I stay at this dose?
  • Abnormal test result questions:
    • Could this condition be genetic? Is it associated to a particular gene or chromosome?
    • Could the cardiac issues be caused by the vascular compressions or vice-versa?
    • Could I still have P.O.T.S. or are my symptoms caused by the cardiac issues?
    • Is there any way to know the cause of the pericardial effusion?
    • Was there a blockage in the arteries?
    • What is the treatment?
    • Why did I only show an abnormal heart rhythm after exercise?
    • Should I continue to salt-load and water-load, even though that is against cardiac treatment but good for P.O.T.S.?
      • Should I still wear the compression socks?
    • Why did these abnormalities not show on any EKG?
    • Do I have sustained or nonstained ventricular tachycardia?
      • Is the tachycardia polymorphic or monomorphic?
    • Is there any evidence that I’ve had a heart attack?
    • Could the ventricular tachycardia be causing the low ejection fraction?
    • Do I have diastolic or systolic heart failure?
    • Could the cardiac issues be causing the gastrointestinal symptoms?
  • Other questions:
    • Will I still go to the Dysautonomia Clinic at University Hospital?
    • Should I get a second opinion from vascular surgery?
    • Could any of this be related to neurotransmitters or hormones?
      • particularly, catecholamine and cortisol levels?
    • What s the likelihood I have an infiltrative disease or autoimmune disease?
      • Amyloidosis?
      • Lyme?
      • Sarcoidosis?
      • Other?
  • Most important question:
    • What is my prognosis?

I had visions of the doctor literally picking me up and throwing me out of the office, shortly followed my “scroll” of questions. I highly doubted that it would actually happen, but you never know these days. It’s was a lot to ask. I typically try to keep both my medical concerns and my list of questions to five or less each, respectively. However, I felt this was super important and I needed to know. I opted to take my chances.

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The night before my follow-up appointment with the cardiologist I could barely sleep.

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There were too many things running through my mind. Do I have everything I need? What am I forgetting? What do I do if he says [this]? What if they tell me [that]? I literally drove myself crazy just over thinking everything. I knew I needed sleep more than anything. It was an hours drive again and I was scheduled first thing at 7:30 a.m. I think I finally fell into sleep somewhere around 3:00 a.m. before my alarms went off at 5:00 a.m. I was too anxious to need much sleep anyway, the adrenaline kept me awake that morning on the drive anyway.

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We arrived at the clinic that morning, early as usual.

It wasn’t long after we checked in that we were escorted back to the exam room. From there, though, we had a wait, which is unusual for this clinic.  We wait in silence as 5 minutes go by, then 10,  and 20. You could feel the anxiety and tension build up in the tiny exam room as we waited on the cardiologist. I could see my husband getting impatient, it’s all over his face. I, myself, feel like I’m going to explode inside. I don’t dare to breathe. Just when I can’t take it anymore, the doctor walks in.

I take a deep breath. My palms are sweaty. My heart is beating. 

I just want to get this over with.


results

Blue=My notes

Red = Abnormal results

The Echocardiogram With Agitated Saline:

Physician Interpretation:

Left Ventricle: No regional wall motion abnormalities noted. The left ventricle
cavity size is normal. Left ventricular systolic function is mildly reduced. Normal left ventricular diastolic function with normal LA pressure. Left ventricular wall thickness is normal. There are false chords noted in the left ventricle

[Had to look this up, as I had no idea what it meant: “Left ventricular (LV) false chordae tendinae (false chords) have been implicated as a source of idiopathic left (IL) ventricular tachycardia (VT). However, it is unknown whether pretest bias contributes to an apparent association with disease. The purpose of this study was to determine the prevalence of false chords on direct inspection of the LV endocardium”.]

Right Ventricle: Global RV systolic function is normal with a tricuspid annular
plane excursion of 1.76 cm. Right ventricular size is normal.
Left Atrium: The left atrium is normal in size with a left atrial volume index of
10 ml/m2.
Right Atrium: The right atrium is normal in size. Eustachian valve seen in the
right atrium (normal finding).

[Of personal note here, I looked up to see why this would be noted like this and I found this:

“Eustachian Valve: It is a remnant of a fetal structure that directed incoming oxygenated blood to the foramen ovale and away from the right atrium.  

Incomplete regression of this structure results in a thickened ridge at the IVC/RA junction, which can occasionally be thick enough to mimic thrombus or a right atrial mass on echocardiography, cardiac CT, or cardiac MRI.

A thickened Eustachian valve may also interfere with placement of an atrial septal defect or patent foramen ovale closure device.

The Eustachian valve can be seen in the 4-chamber view or the bi cavil view of the right atrium; it is seen in approximately 25% individuals, at the junction of the IVC and right atrium. It appears as an elongated, membranous, possibly undulating structure. Usually it is of no physiological consequence, but can be confused with an intracardiac thrombus, cause turbulent atrial blood flow, complicate IVC cannulation or serve as a site for endocarditis formation”.]

Aortic Valve: The aortic valve was not well visualized. No evidence of aortic
regurgitation is seen. No evidence of aortic valve stenosis.
Mitral Valve: Trace mitral valve regurgitation. No mitral valve prolapse is noted.
Tricuspid Valve: Grossly normal. Unable to estimate Right Ventricular systolic
pressure due to inadequate or absent TR Doppler signal. There is trace tricuspid
regurgitation.
Pulmonic Valve: The pulmonic valve was not well visualized. No pulmonic valve
stenosis. Trace pulmonic valve regurgitation.
Vessels: IVC is normal in size with normal inspiratory collapse suggesting a normal
right atrial pressure (3) rnrnHg.
Aorta: The aortic arch was not well visualized. Aortic root is normal in size. No
obvious coarctation of the aorta noted by 20, Doppler.
Pericardium: No definite echocardiographic evidence of hemodynamic compromise. There is a moderate pericardial effusion localized near the right ventricle.
Shunts: There is no obvious right to left shunt at rest, with cough, or Valsalva on
agitated saline contrast examination.

The Holter Monitor:

1. Sinus rhythm, predominantly sinus tachycardia, with rates between 61-190
bpm and average rate 101 bpm .
2. Supraventricular ectopy: One isolated PAC in 24 hrs.
3. Ventricular ectopy: One, isolate 7 beat run ~f monomorphic ventricular
tachycardia with irregular rate 169 pm at 11:59 AM; otherwise, no other
ventricular ectopy.
4. Longest R-R was 1.2 seconds during sinus arrhythmia.
5. Symptoms of “fatigue, faint, abdominal pain, dizzy, chest pressure, chest
pain, flutter, chest tightness, pre– syncope,” and patient events all correlated
with sinus tachycardia, and in particular, the  symptom of “pre-syncope” correlated with sinus tachycardia 185 bpm; with the  7 beat run of monomorphic ventricular tachycardia was asymptomatic.

The Exercise Stress Test:

Summary:

1. Fair age- and gender-adjusted exercise capacity.
2. No evidence for exercise-induced ischemic ECG changes at the level of
exercise achieved.
3. Normal HR response (patient held Metoprolol for 48+ hours prior to exercise,
normal BP response. Target heart rate was achieved.
4. Pulse oximetry readings were greater than or equal to 95% on room air
throughout -the study.

Note: The baseline ECG reveals sinus tachycardia, rate of 107 bpm. ST-T shifts of ischemia or ectopy noted.

The Cardiac MRI w/ and w/o Contrast:

RESULT: Cardiac MRI
Clinical History: Pericardial effusion, cardiomyopathy. Evaluate LV function
delayed enhancement pattern
Technique: Following initial axial haste images, cine and dark blood images were
obtained in short axis, and vertical and horizontal long axis. 20 ml of ProHance
were administered intravenously, without adverse event. Immediate images were
obtained for perfusion. Delayed images were obtained in all 3 planes to evaluate
for delayed hyperenhancement. VIBE sequence was additionally acquired through the
lungs.
Cr: 0.95
eGFR: 72

The National Kidney Foundation (NKF) suggests only reporting actual results once values are < 60 mL/min (they state normal values as 90-120 mL/min). An eGFR below 60 mL/min suggests that some kidney damage has occurred.

KIDNEY DAMAGE STAGE DESCRIPTION GFR OTHER FINDINGS
1 Normal or minimal kidney damage with normal GFR 90+ Protein or albumin in urine are high, cells or casts seen in urine
2 Mild decrease in GFR 60-89 Protein or albumin in urine are high, cells or casts seen in urine
3 Moderate decrease in GFR 30-59
4 Severe decrease in GFR 15-29
5 Kidney failure <15

Findings:
Survey images of the mediastinum show normal heart size. There is no pathologic mediastinal adenopathy or pleural effusion.
Atria: Right and left atria are normal in size and contract normally.
Right Ventricle: Right ventricle is normal in size. Globally preserved systolic function is preserved. No wall motion abnormality.
Left Ventricle: Normal size and wall thickness. Globally preserved systolic
function without wall motion abnormality.
Pericardium: Small pericardial effusion without evidence of constrictive physiology.

Perfusion images show: Homogenous perfusion without focal abnormality.

Delayed hyperenhancement images show: No delayed myocardial enhancement. Apparent focus of increased signal intensity seen at the lateral aspect of the base appears most consistent with a focus of epicardial fat when comparing to SSFP images and four-chamber and short axis sequences ( four-chamber series 3 image 59, short axis series 57 images 1 through 3) .

Left ventricular ejection fraction: 59%
End diastolic volume: 84 ml
End systolic volume: 34 ml
Stroke-volume: 50 ml
Cardiac output: 4.2 liters per minute
Left ventricular myocardial mass (at ED): 59 g
Right ventricular ejection fraction: 51%

IMPRESSION:
No delayed myocardial enhancement to suggest infiltrative cardiomyopathy.
Preserved LV systolic function without wall motion abnormality.
Small pericardial effusion without evidence for constrictive physiology.


INTERVAL HISTORY:
Nichole returns following her initial visit with me on 07/08/2015. At that time, we had performed an echocardiogram to assess for LV size, systolic function and possible pericardial effusion. This revealed the presence of a moderate size effusion without clear evidence of hemodynamic compromise. Also surprising was the presence of borderline reduced LV systolic function.. Based upon these findings, a cardiac MRI was run and revealed preserved LV systolic function, EF 59%, with normal left ventricular end diastolic and systolic volumes. RV ejection fraction was also normal at 51%. There was a small pericardial effusion which was circumferential and without clear evidence of septal shift or other stigmata of constrictive physiology. Also surprising was the presence of what was identified to be a 4-beat run of wide complex tachycardia which occurred at a rate of 169 beats per minute at 12:00 a.m. There were no associated symptoms. There were multiple entries recording complaints of fatigue, faint abdominal pain, dizzy, chest pressure, chest pain, flutter, chest tightness and presyncope, all of which were correlated with sinus tachycardia. Based upon these findings, we start Nichole on metoprolol XL 25 mg daily, which she initially felt somewhat more fatigued and dizzy on, but since that time has adjusted to. She has also made a more concerted effort to use volume and sodium loading, for which she feels better overall from a POTS standpoint. She continues to report left-sided chest pain, which is not necessarily positional in nature.

REVIEW OF SYSTEMS:
Positive for weight gain, fatigue, loss of appetite, chills, dizziness, nosebleed, shortness of breath, chest pain, palpitations, near fainting/fainting, leg cramps, abdominal pain, diarrhea, constipation, nausea, vomiting, blood in stool, rash, itching, nighttime urination, snoring, back pain, muscle aches and joint aches. Comprehensive review of
other 12-organ review of systems is otherwise negative.

ALLERGIES:
Epinephrine caused adverse reaction.
IMPRESSION:
1. Pericardial effusion in the context of presumed autoimmune disorder not otherwise specified, possibly lupus with negative antinuclear antibody (ANA), undergoing further evaluation with [Immunology]. Pericardial effusion does not appear to be associated with constrictive physiology by echocardiographic criteria. This is likely chronic in
nature. I cannot exclude the possibility of chronic pericarditis as a contributing cause to her chest discomfort.
2. Probable postural orthostatic tachycardia syndrome (POTS), complicating #1.
3. New onset wide complex tachycardia, possibly ventricular tachycardia. I cannot exclude atrial dysrhythmia with aberrancy tolerating beta blocker therapy, with associated preserved left ventricular (LV) systolic function by magnetic resonance imaging (MRI), which is gold standard data for ventricular volumes and function.
4. Prior history of superior mesenteric artery (SMA) syndrome and May-Thurner syndrome.

PLAN:
1. Referral to Dr. [Vascular Surgery] at the University cardiovascular center for a second opinion.
2. Consider initiation of low-dose ibuprofen. I will discuss this plan of care with Drs. [gastroenterology] and [immunology] to ensure that this is appropriate from their perspectives.
3. Continue beta blocker therapy for ventricular tachycardia versus supraventricular tachycardia (SVT) with aberrancy with monitoring symptoms.
4. Return to clinic in three months’ time for clinical reassessment.
5. Repeat echocardiogram in three months’ time for reassessment of pericardial effusion, particularly should we initiate nonsteroidal anti-inflammatory drug (NSAID) therapy.
6. Avoid prednisone therapy due to potential provocation of a relapsed pericarditis.
7. Collaborative care with Drs. [gastroenterology] and [immunology].


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The good news: My heart is not failing like they originally thought on the left side. In fact, my ejection fractions are higher on the left than the right.

The bad news: Looks like there are some valvular problems with blood flow, but only mildly. Also, there are a few structural and/or congenital abnormalities which have led me to some further research that is quite interesting, but I won’t post until I’ve got a few more pieces together and have talked to the doctor about it.

Finally, there’s no significant answers or treatment plan at this time. At this point we are continuing medications as previously prescribed. Both the GI and the Immunology doctors gave the go ahead to start 200mg Ibuprofen twice a day (although right before this test I was on 800mg ibuprofen one time a day for bleeding following my endometrial ablation – which means it probably won’t reduce the pericardial effusion, but we’ll see).

The cardiologist does not want to start me at the dysautonomia clinic until he is sure that the effusion is not contributing to my POTS symptoms, although he doubts that it is. He still firmly believes I have P.O.T.S. or some form of dysautonomia, but he doesn’t want to send the referral just to get sent right back over something he should be handling on his own anyway. Still no idea what could be causing the pericardial effusion, but the cardiologist continues to believe it is something autoimmune related (due to its chronic nature), despite what the immunologist now says about having zero indications for autoimmune disease (even though that wasn’t what she told me) but not sure if we’ll ever find it if it is. Did a random skin biopsy on my arm last week when I had a “vasculitis-type rash”, but it came back inconclusive as well. I should know more when I get the report when I get the sutures out of Tuesday.

The cardiologists did, however, give me a referral for a second opinion from vascular surgery at University. I think he has some ideas on some of the research I am contemplating as well, but he won’t say at this time. He put my order in as “Urgent”. The new hospital called a couple of days later to schedule, but I couldn’t get the other hospitals to send records and scans over it time for the appointment. We rescheduled with them for this upcoming Tuesday. I’m hoping they can shed more light on the impact of the compression disorders or, at the very least, believe they exist (which they do).

It’s been a whirlwind couple of weeks as far as medical stuff goes. While I’m tired and ready for a break, at least we’re getting somewhere and I’m not going to die like I thought after the first few phone calls from the cardiologist’s office. My heart is not “normal”, so it’s not good news but it’s not bad either. So far, the Metoprolol is not helping the tachycardia, but I am still on a low dose and I really need to call the doctor and see if we can do an increase. So while this is the end of the chaos with cardiology, at least for now, it’s only the beginning for so many other new doctors and appointments.

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Welcome to the new age, to the new age…

The anticipation had been killing me, but I finally received the call that I had been eagerly waiting for from the vascular surgeon. I was in my therapist’s office when the call came in, but luckily she was kind enough to let me take it since she knew it was something I had long been waiting for. I think she was curious herself. Needless to say, it was probably a good thing I was in her office when the call came in, as I was not a happy camper.


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He started by saying he looked at my CT Angiography results and he had spent some time doing research, as well as talking to fellow vascular surgeons. 

Good.  I figured he had some research to do, considering the result. 

He goes on to say my renal vein definitely shows a large amount of compression.

Okay, go on.

He asks how I’ve felt lately.

“Terrible.”

“Remind me of your symptoms again?”

*sigh*

“Well, I have had horrendous stomach pain every day.

I’m nauseous constantly.  

I can’t eat anything without being sick. I don’t digest my food.

I have diarrhea and/or constipation that is limiting.

I am tired all the time. And not just tired, EXHAUSTED.

Dizzy, heart palpitations, spinning, and chest pain.

My limbs go numb. I have arthritis. My legs are stiff and painful.

I have livedo reticularis. 

I have painful rashes. They are getting worse and change day-to-day.

My hands are now so blistered I can barely hold on to anything…”

He says, “well none of those explain Nutcracker Syndrome…”

“Right. I haven’t had hematuria in years. Just the back pain and left, flank pain. Abnormal, unexplained bleeding. But all my abdominal pain is on my left side…

“So yeah, nothing relating to the Nutcracker Syndrome. Like I said before, I don’t believe in it.”

“What about the Superior Mesenteric Artery Syndrome? Or the May-Thurner? Both were noted in the results.

“I saw it, but I don’t believe they are the cause of your symptoms” he says.

“Well, SMAS can cause severe abdominal pain, nausea, anorexia, vomiting…”

” No vascular compressions typically causes any symptoms at all. None of us (vascular surgeons) believe that these ‘syndromes’ exist.”

“Oh…”

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“Besides, on the small chance that these conditions even exist, they are so rare. It’s not possible for you to have all three. “

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So the report it wrong? Because it says I have all three.

I felt my face getting really red. I didn’t know whether to cry or scream.

“I think whatever you have is systemic.”

“But it’s not showing in my blood work. If it was systemic, you’d think my blood work would show it…”

(I think this to myself, but I can’t say it out loud. I’m too frustrated and  taken off guard to even say almost anything at all)

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“Like I told you during the consult, I want to take a very ‘conservative approach’ to this. I don’t suspect the Nutcracker syndrome is causing your illness. I suggest you continue looking for the cause of your illness elsewhere. After you have exhausted everything else possible, then come back and I’ll re-examine you again. If at that time, you don’t have a diagnosis despite exhausting all other options, then we can go ahead and try to balloon the vein and see if there is any relief.”

“Okay. Thanks for calling.” I hang up.

I’m positive that there was no hiding the disappointment and  sadness in my voice during that call.

I don’t care though. Maybe it’s something he should hear. Not that he cares.

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I am beside myself at this point. I can barely breathe, my chest is tight, and I have no idea what to do.

So let me get this straight…

All these world-renowned doctor’s, who have researched these disorders extensively, don’t know what they are talking about?

The Mayo Clinic, The Cleveland Clinic, and The NIH know less than you, is that correct?

All the patients in my support groups are just lying about their symptoms?

Everyone in the Nutcracker Syndrome Group?

The Superior Mesenteric Artery Syndrome Group? The Wilkie’s Group? SMAS Support?

The May-Thurner Syndrome Group as well?

Not to mention these patient’s doctors (world-wide) who not only diagnosed them with these “imaginary” syndromes but also are treating them for it too. Are they lying?

The invasive surgeries that these patients have undergone, their feeding tubes that have saved their lives, their medications… Is it all for nothing?

All of those people with these ‘non-existent’ disorders, particularly SMAS, that have lost their lives to these conditions… must have never existed either, right? 

What a crock of sh*t!

{Excuse my language.}

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I debate on sending him all the research and case studies I have found.

There’s so many, but it probably wouldn’t matter anyways.

GARD Superior Mesenteric Artery Syndrome

Superior Mesenteric Artery (Wilkie’s) Syndrome as a Result of Cardiac Cachexia

Wilkie’s Syndrome

Wilkie’s syndrome causing persistent vomiting

Patient with Both Wilkie syndrome and nutcracker syndrome

Nutcracker Phenomenon and Nutcracker Syndrome

Case Report: Nutcracker syndrome: A rare anatomic variant

Current trends in the diagnosis and management of Renal Nutcracker Syndrome: A review

May-Thurner Syndrome

The list goes on and on…

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I feel panic inside. Now, what do I do? Where do I go from here?

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I tell my therapist what was said. Of course she thinks this is crazy. At least it’s not just me. She has looked into every condition and every medication the doctors have mentioned or I’ve found in my research. This is why I like her so much and why I continue to keep going. She tries to understand me. She respects my opinions. At least there’s someone.

I see her look down at my hands and legs. My hands are red, swollen, peeling, and visibly painful. My legs clearly look bruised in spots, with purplish-molted discoloration.  

She asks me, “what’s next?”

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I have composed myself by this point. No point in breaking down, anyway. There’s nothing I can do to change this doctor’s mind. Plus,  I’m used to being disappointed by doctors. He’s not the first one to not believe me, or give up on me. Sadly, he probably won’t be last either. 

“I find another vascular surgeon. Get a second opinion. I start over… again.”

“How do you feel about that?”

“I’m just tired of it, really. I feel nothing anymore. You get so close, then hit a wall. Over and over again.”

“That’s frustrating. It shouldn’t be that way”

“No, it shouldn’t. It’s unfair and it sucks, but that’s the reality of it. This is how it is to be chronically sick. Normal people don’t see this side of healthcare in their yearly check-ups or their infrequent appointments for a cold or flu. But we (those with chronic illness) deal with it every day.  Every day is a fight to get the proper care. These doctor’s don’t care. Health care in the United States was not meant for us (chronically ill). Yet, we keep fighting, who know’s how. Probably because there is no other choice. What else can you do?”

I lean further back in my chair. I’m not hiding my frustration or disappointment well. I know she understands, or at least can acknowledge,  how I feel. This alone makes me feel better. Well, at least better enough not to scream or cry, which was my initial reaction to the news. Even though I have had plenty of time to process what was said by now, I’m still in a bit of shock.

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As per usual, today was spent picking up all the pieces.

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I called multiple vascular surgeons all over the entire state and I found a few actually in town that accepted my insurance. Although I know an in-state doctor more than likely won’t have a great deal of expertise on any of my conditions, it’s the only option I have currently. I’d prefer to see one of the very few doctors (all in the highly accredited and well-known clinics across the country) that specialize in these disorders, but my insurance won’t pay out of state and it’s just not feasible for me financially. At least not at this time, maybe ever.  

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I made sure to ask them if they had surgeons who were familiar with ‘rare vascular compression disorders’. Of course, a lot of explaining of the conditions ensued and I had to be placed on hold multiple times while they asked the doctors, but I found two potential offices that may be able to at least give me a second opinion. They need another referral, however, which wouldn’t be an issue if my primary care office would actually answer their phone so that I could schedule an appointment with my doctor. But alas, both yesterday and today I got the infamous voice mailbox that literally goes nowhere. (There’s no actually recording, just a beep to leave a message. And every time I do leave a message, I NEVER get a call back) I guess I’ll try back on Monday.

Thankfully, Monday is the start of a new week.

It’s a good day to start from the beginning, once again.

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Oh doctor, doctor, I must have gotten this sick somehow. I’m going to ask you a series of questions, and I want them answered on the spot, right now.

Patience has never been a virtue of mine, as many of my friends and family can attest to.

But I feel that I have been extremely patient with my doctors, maybe more than I should be.

I wasn’t planning to do an update until I received the final confirmation from my doctors,

but it seems like that won’t be happening anytime soon,

so I figured that no time is better than the present.

It’s been just about a month since my abnormal tests came back. I have left multiple messages for both doctors, one every week, hoping for a callback or at least an idea as to whether or not I need to come in for an appointment to discuss the test results in person.However, all I got was silence. I literally felt my blood boiling each day that went by that I didn’t receive a call. 

I’d whine about how:

“They’re delaying my care.”

“Sure, just because I’ve been sick forever, no reason to rush or anything.”

“I don’t know how doctors can get away with not calling when tests are abnormal.”

And, of course, the classic:

“I’m going to die before they ever figure out what this is.”

I was feeling sorry for myself, to say the least. I felt alone, abandoned, and lost as to what to do next. It was really starting to mess with my head. I hated being this way but I hated how these doctors were making me feel even more.

I felt like they were completely disregarding everything I have gone through

in my search for a diagnosis.

The years of life I have lost due to being sick.

The hours spent driving to appointments.

The amount of time in hospitals and doctor’s offices.

The high levels of radiation and all the discomfort in medical testing.

The countless pills prescribed, which often caused more side-effects than actual relief.

The procedures and surgeries, which also didn’t fix my disease.

The friends and family I lost, because they didn’t understand.

My career goals and aspirations that have been placed on hold.

Everything I once was and what I could be.

My whole life is on hold!

And here, right in front of you, are these abnormal tests

that provide answers to my chronic, undiagnosed illness

that I’ve fought so hard, for so long, to find

and you can’t pick up the phone to call me? 

Maybe I am being impractical,

but a month spent waiting seems way too long.

That’s a month to complete more testing or get second opinions.

Four weeks of treatment or management.

30 days of my life lost, left waiting.

FINALLY, a nurse from my GI, Rheumatologist/Immunologist, and Cardiologist’s office called, but she only caused  more confusion and frustration than before. She tells me my fecal cultures came back normal, which I already knew. She then says my cortisol levels were low (again, I’ve had my test results for weeks) and the doctor would like me to rerun it to be sure. She’s going to mail the paperwork so I can at least have my blood drawn in town, instead of driving over an hour. Great, thank you. She also tells me to stop taking my lupus medications… wait, what? The GI doctor and the Rheumatologist/Immunologist agree that I should stop taking them, because I’m not autoimmune. Um… ok. How come she originally, as did all the other doctors, think it was seronegative autoimmune disease? Specifically, Lupus?  I’m not in the mood to argue, I’ve been feeling terrible. I ask if I stop them forever, she says yes and the conversation is over.

She calls again yesterday, just to tell me to stop taking the Lupus medications… again. Yes, you told me the day before. Both doctors think it may be causing my painful and urgent diarrhea.Um, I have had on and off urgent diarrhea for… like… ever. But ok. So it’s not because it can’t possibly be autoimmune? I’m confused. She asks if the medication has helped. I said I’m over the initial side effects. I’ve had constipation for the past few days, so don’t think it’s causing diarrhea, and I haven’t had any extreme photosensitivity as in the past weeks, but still having outbreaks of rashes. I don’t know what is causing what anymore. My symptoms are too random and sporadic. She says to call next week or the week after, let them know if being off the medication makes me feel better or not, and then we will restart it if it’s not the problem. Wait… so let me get this straight? Now you’re taking me off it, to see if it’s having side effects I have had forever, only to restart it and have to adjust to it once again? Are you kidding me? Well, it may be affecting your cortisol levels, so they want to see if I stop it if it’ll change my blood test.  I am beyond confused and frustrated at this point. I went and redid my cortisol blood test this morning, so I guess we’ll just wait and see what that tells us.

Still haven’t gotten a call from the vascular surgeon. My CT Angiography was the one I was MOST worried about and  I fretted every night about him not calling me to discuss the findings. . Finally fed up, having left four messages now, I called and scheduled an appointment to discuss. I don’t know whether or not I trust him to treat at this point, given the lack of respect of promised phone calls with no answers, but he may just not know what to do or say about the finding. This is the doctor who didn’t believe in Nutcracker Syndrome at all for his own, valid reasoning, but admitted my original CT Scan showed the most convincing case of Nutcracker Syndrome that he has seen in over 30 years. He ran the CTA to “prove him wrong”. On the order form for the test, he even wrote “to exclude Nutcracker Syndrome”, instead of “evaluate for Nutcracker Syndrome”. He was really convinced I couldn’t have it, it’s too rare, and most vascular surgeons don’t think it’s a real thing.

Well, guess what? I proved him wrong. Not only that, they found two more (even rarer)  vascular compressions. The radiologist noted both May-Thurner Syndrome and Superior Mesenteric Artery Syndrome, although the Nutcracker Syndrome is the most extensive. So perhaps, maybe he is lost as to what to do or say at this point, I don’t know. But I’d rather him tell me that if that is, in fact, the case, rather than be silent about it. But I have an appointment now, so he can’t ignore me. So we’ll see how that goes.

I also had my consultation with cardiology last week. It had gone way better than expected and I really liked the doctor. He not only listened to me, he caught things other cardiologists had  missed in the past, and had my notes completed (and accurate) by the end of the day. I was fearful for this appointment, as I have not had the best luck with cardiologists in the past. They always say they hear a “murmur” or “valve issue”, order tests, and then call me crazy. This has happened on multiple occasions, both in my teens and early twenties. So you can see how I’d be nervous about going straight into an appointment saying “I think I have POTS syndrome and so does my neurologist and the immunologist (although she seems to have forgotten EVERYTHING she told me in my last appointment, so maybe she doesn’t think I do anymore, who knows)”. I show him the letter from the neurologist and my ‘poor man’s tilt table test’ results. He says that it looks like I have POTS, but he wanted to have some “orthostatic fun” in the office just to see. He measured my heart rate and blood pressure while laying down, sitting up, and standing.  Sure enough, my blood pressure dropped really low and my heart rate increased up to 150. Yep, he’s pretty convinced that it is POTS, but because of the missed information in previous cardio tests, he wants to rerun them again just to make sure it’s not something “easier” or misdiagnosed.

In my echocardiograms from 2005 and 2007,  he noticed that there was what he called “abnormal electricity” shown, but the EKG didn’t catch it, so it was dismissed. It happened again in my 3D echo from last year. Also, the 3D echo from 2014 showed I had pericarditis, which is a typical sign of autoimmune (particularly Lupus), but, of course, need to rule out other possible causes as well. And finally, my halter monitor from 2007 showed abnormalities and heart beats exceeding 160 bp, which was also dismissed during that time. So he ordered a 3D echo again, to see if the pericarditis has cleared on its own or if it’s gotten worse. He also wants to evaluate the possibility of a hole in my heart (which many people are born with, although it usually clears up on its own as you get older) since they can’t confirm the cause of my hypoxemia, other than the mild sleep apnea that was confirmed through my sleep study last year (although he doesn’t believe that is what is causing it, because again, it was very mild and happens sporadically during the daytime as well). So I’ll be back in the hospital tomorrow to complete all of these cardio tests. If  all the differential diagnoses are excluded, then he will be referring me to the dysautonomia clinic for further treatment, but was comfortable enough to put down Postural Orthostatic Tachycardia Syndrome as an official diagnosis. Again, something I have been saying since I started looking for answers. Finally! So we’ll see how testing goes tomorrow and I guess go from there.


So what does this all mean?

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It’s more than just ONE cause, obviously.

There are multiple conditions feeding off of one another, 

making my conditions not only rare,

but also complicated to treat and manage.

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Here’s what is on the table (so far):

Dysautonomia:

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More specifically —> Postural Orthostatic Tachycardia Syndrome (POTS):

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Nutcracker Syndrome:

“The nutcracker syndrome is quite a rare condition. It is due to the compression of the distal segment of the left renal vein (LRV) between the superior mesenteric artery (SMA) and the aorta (also called left renal vein entrapment).  This syndrome needs treatment when symptoms are disabling” (Hartung, O., 2009, p. 246).

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Superior Mesenteric Artery Syndrome (SMAS):

“Superior mesenteric artery syndrome (SMAS) is a digestive condition that occurs when the duodenum (the first part of the small intestine) is compressed between two arteries (the aorta and the superior mesenteric artery). This compression causes partial or complete blockage of the duodenum. Signs and symptoms may include abdominal fullness; bloating after meals; nausea and vomiting; and abdominal cramping that may be helped by lying in certain positions.” (NIH Office of Rare Diseases, 2014)

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May-Thurner Syndrome:

“May-Thurner syndrome (MTS) is caused when the left iliac vein is compressed by the right iliac artery, which increases the risk of deep vein thrombosis (DVT) in the left extremity. DVT is a blood clot that may partially or completely block blood flow through the vein. Even though DVT itself is not life-threatening, the blood clot has the potential to break free and travel through the bloodstream, where it can become lodged in the blood vessels of the lung (known as a pulmonary embolism). This can be a life-threatening condition” (ClevelandClinic.org)

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Symptoms and Causes of Low Cortisol Levels:

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A lower than normal level may indicate:

  • Addison disease, in which the adrenal glands do not produce enough cortisol
  • Hypopituitarism, in which the pituitary gland does not signal the adrenal gland to produce enough cortisol
  • Suppression of normal pituitary or adrenal function by glucocorticoid medications including pills, skin creams, eyedrops, inhalers, joint injections, chemotherapy

Other conditions for which the test may be ordered include:


Is this a final diagnosis?

Knowing how thing have gone in the past, it’s  highly doubtful.

Maybe there is more to the story…

Maybe there is less significance…

Only time will tell.

Again, I am left waiting…


Now if only I can get all my doctors organized and working together, maybe I can clear up what this all means and what needs to be done next. Surgery? Medications? Again, who knows… 

While I DO know for sure that NONE of these diagnoses will ever be “cured”, I’m hoping we can at least find a way to manage everything so I could hopefully live a semi-normal life again.

I still have to do a hydrogen/methane breath test next week, as well as upcoming appointments the week after with dermatology (to run biopsies on my skin rashes, hopefully to “catch” the autoimmune disease that’s hiding in my skin) and a follow-up with the vascular surgeon. Plus the results from the cortisol testing I did today and the cardio tests tomorrow. Let’s hope we can get the pieces put all together and figure out what’s next as far as treatment goes.

I guess I’ll just have to wait… something that is unfortunately becoming entirely too common at this point, but at least we’re getting somewhere… slowly. 

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Just stand still, look pretty.

But you don’t look sick…

My illness is only invisible because I decide what you can and can’t see.

Much of living with chronic illness is hidden from the outside world,

in an attempt to be as normal as physically possible.

So unless you live it yourself, you’re blind to it.

But nothing is truly invisible if you make a conscious choice to really open your eyes.

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So what does invisible illness really look like then?

Allow me to show it you.


You can see invisible illness in the things I do each and every day.

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Visible in the adjustments I make just to do everyday things.

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You can see it on the pages of my planner in which I write every appointment and daily to-do list.

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Or in my notebook that I take everywhere with me.

Otherwise, I will forget everything.

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Or the time I spend doing medical research,

hoping to find an answer for what the doctors can not find.

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You see it in the amount of caffeine I drink, just to stay awake.

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In how long it takes me to do the housework and laundry.

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Or how hard it is just to run simple errands.

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You can see it sitting on my bookshelf.

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In number of times I wash my hands in a day.

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Or the fact that a good portion of my time is spent hidden away in a bathroom.

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Illness doesn’t leave room for hobbies, much less the things that are fun.

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Invisible illness is seen in the never-ending doctor’s appointments and medical testing.

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Or the procedures I’ve had, despite knowing whether they will work or not.

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You see it in all the blood draws the doctors run regularly, trying to get a diagnosis.

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And the therapy appointments I attend just make sure I am not crazy.

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You see invisible illness in all the paperwork I have to complete and keep track of.

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In the two three inch binders that hold my medical records

which I need to bring to every doctor’s appointment.

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-one for clinic notes, one for labs/testing-

You see it in the summaries I put together to keep all my doctors on the same page.

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Or in the advanced directives, living wills, and Do Not Resuscitate orders.

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My illness is clearly visible in the medications I take  every day.

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You can see it in my oxygen concentrator and tank that help me to breath.

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In the duo-nebulizer that I keep at home

just in case an attack comes on and I can’t get to the clinic in time.

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 In my monitoring tools -in my blood pressure cuff and oximeter.

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In my heating pad and humidifier.

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My invisible illness is hidden deep inside my travel case,

full of emergency medications and supplies for when I leave the house.

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And in the lessons my husband has had to take to administer medication

or help me in case I can’t help myself.

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You see my invisible illness in the symptoms I try to conceal and hide.

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Just because it’s not easy to see, doesn’t mean it’s not there.

Do I look sick enough now?

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Because this is how I look at home, behind closed doors.

In real life, invisible illness is not so invisible afterall.

Remember that the next time you judge someone,

when you don’t believe they are as sick as they make out to be,

when you make them prove how sick they truly are,

or say “but you don’t look sick…”

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Keep Your Head Up, The Colors Are Beautiful

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Lot’s of new things on the horizon, so I figured I’d give a short update.

  • On UndiagnosedWarrior: Been updating and organizing pages, adding new information, noting some tips and tricks, and other things here and there. Take a look around and let me know what you think. I have some good ideas and really want to add more content for those looking for a diagnosis, as well as those who have already been diagnosed. And, of course, keeping you updated on my search for a diagnosis. 
  • On Life: I’m officially back out of work, but this time my short term disability has been approved. After the whole mess with getting a Lupus diagnosis, then having it taken away, then given back by a different doctor, I’m still confused as to whether or not I ACTUALLY have it.  I have a few tests pending and some recent tests that have come back with very interesting results (*Hint: It was enough to finally PROVE my disability claim). I’m waiting for the doctors to call to discuss their thoughts, so I don’t want to jump the gun on yet another diagnosis, so I’m going to wait to post, but I’ll update as soon as I can.

As for now,

I just want to leave you with the strength in knowing

 that all your struggles, all your hard work, and your persistence to keep looking 

IS WORTH IT!

I know it is hard to stay patient and that you are tired of waiting,

especially when  you have been sick for so long.

But waiting is always the hardest part. 

Keep trusting your instincts. 

Trust the journey.

You know your body better than anyone.

The answers lie WITHIN YOU.

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“Keep your head up
The colors are beautiful
When they say give up
Turn up your radio
All the sentimental memories you own
When they say grow up
It’s just like a funeral
Keep your head up
The colors are beautiful
Keep your head up
It’s all right in front of you
When they say wake up
You’re breaking ridicule
When all the sentimental memories you own
Keep you trapped inside your room there all alone

And it feels like
It feels like you’re lost
And it feels like
It feels like you’re lost

Is there some way you can be out on your own?
Trust yourself
Don’t waste another day at all

On your own

Keep your head up
The colors are beautiful
And it feels like
It feels like you’re lost
And it feels like
It feels like you’re lost

Is there some way you can be out on your own?
Trust yourself
Don’t waste another day at all

Watch this fade away
Everything fades away
Keep your head up
The colors are beautiful”

(“Head Up” by Sugarcult)